Following my diagnosis of idiopathic pulmonary fibrosis (IPF) in 2016, I was told by my physicians that this disease could progress in one of two ways. The first was that the fibrosis would progress rapidly and leave me short of breath and fatigued with a chronic cough. The second option the doctor discussed was the IPF progressing slowly and potentially leaving me stable for a number of years where I’d experience very little changes to my physical abilities. Of course, as a young adult who was otherwise physically active and healthy; I prayed for the latter.

Learning to live with IPF has been one of the hardest things I’ve had to do in my young adult life thus far. It has been three years since my diagnosis, and overall I’d say the progression of this fatal lung disease has been slow, which I am grateful for. While I do experience some difficulty in doing certain tasks now, others I am still able to manage with supplemental oxygen or assistance from friends or family members. That said, in the last several weeks I’ve been forced to accept help from those around me as I’ve dealt with a number of illnesses that have worsened the progression of my disease.

What is an acute exacerbation of IPF?

According to the Journal of Thoracic Disease, an acute exacerbation of IPF is a sudden acceleration of the disease leading to a significant decline in lung function. Acute exacerbations (AE’s) can be triggered by a known cause such as an illness or environmental factors(s) and unfortunately, AE’s also occur occasionally without any known cause. I experienced my first AE as an IPF patient thirteen months after my diagnosis, which led to an intensive care unit (ICU) admission. I now take as many precautions as I can to stay healthy and avoid cold and flu. Unfortunately, despite my best efforts, this isn’t always possible.

How is getting sick different when you have IPF?

Throughout the past several weeks, I’ve been battling pneumonia and influenza A. These illnesses put me in the hospital and left permanent damage to my lungs. While I am now on the mend, albeit slowly, there is no denying that this setback has affected me in multiple ways. Regardless of why you experience a setback as an IPF patient, whether it is caused by an exacerbation, illness or just physical regression in general, the result can devastate a patient and leave them worried about what their new normal will be. The fear of how my latest setback will permanently affect my lungs has been overwhelming at times.

Below are some of the ways I’ve been affected by this latest setback of having both pneumonia and influenza:

Emotional impact: I regularly try to practice gratitude and being thankful despite my IPF diagnosis; not, however, these last few weeks have left me feeling overly emotional and upset. It is hard to sleep in the hospital due to the constant noise of machines or nurses shuffling around, and I am typically a lot more emotional when I’m tired and haven’t slept well. More than all of these things, however, is the emotional turmoil I’ve been feeling as a result of flashbacks from my ICU admission and the trauma associated with this experience.

Physical impact: I am hopeful that the physical regression I’ve experienced as a result of these illnesses and being immobile is not permanent. However, I notice a significant difference in my stamina and physical abilities already, even as I continue to get well. I am far more exhausted than I’d ever been since my diagnosis of IPF three years back, and I’m requiring a lot more sleep and rest between physical activities. Unfortunately, my lung function has been significantly affected by these illnesses as well, and I’ve experienced an 8% drop in function (FEV1 specifically) and a decrease in my DLCO. For many IPF patients, a loss of lung function is irreversible, and I am that much closer to lung transplant evaluation and listing as a result of getting so sick.

Social impact: Being in the hospital can be very isolating, and I’ve chosen to further isolate myself from social situations and gatherings to promote my continued healing. I’ve been on multiple rounds of antibiotics, antifungal medications and steroids to try to rid my body of the infections from pneumonia and influenza. Unfortunately, each time I think they’re gone, they tend to resurface. I finished all my prescription medications before celebrating my birthday with friends in early December, but it was likely at that very event that I fell ill again, with influenza A virus. I can’t help but wonder if exposing my already compromised immune system to others who may be carrying illnesses without being symptomatic is the reason I keep going backwards in my recovery. It feels like as soon as I get well, I’m hit with another type of virus or bacteria. Consequently, I’ve chosen to stay away from others until I am completely infection-free, which is very socially isolating — especially because I love spending time with my friends.

Financial impact: IPF is a devastating disease for many reasons, one of which is the financial implications of living with fatal lung disease. During the acute phases of my illness, I had to be in the hospital utilizing a number of breathing supports because I couldn’t maintain my own oxygen saturations. While I am now at home recovering, it’ll be months before I can return to work which has a significant impact on me financially. I am lucky to access disability benefits through my employer, but a fraction of my salaried pay. Despite having a reduced income, my bills and expenses including the costs of medications remain the same. Sadly, there is a financial impact on patients when they fall ill or experience an acute exacerbation. This leads to a vicious cycle of being both physically and emotionally burdened by a setback with IPF.

I was always told about the importance of staying as healthy as possible as a patient with a chronic lung condition. This is because of the serious implications that getting sick can have on someone living with IPF, who already has compromised lungs. Not only can it take significant time to recover from a setback resulting from an AE or illness, but also it can easily cause permanent damage to the lungs of an IPF patient which negatively impacts them in various aspects of their lives.

Have you ever dealt with a serious setback as a patient living with IPF? If so, what helped you deal with it?

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